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A case of Schnitzler’s syndrome without monoclonal gammopathy successfully treated with canakinumab by Fujita, Yuya (author);Asano, Tomoyuki (author);Sakai, Akira (author);Norikawa, Natsumi (author);Yamamoto, Toshiyuki (author);Matsumoto, Haruki (author);Sato, Shuzo (author);Temmoku, Jumpei (author);Yashiro-Furuya, Makiko (author);Matsuoka, Naoki (author);Watanabe, Hiroshi (author);Migita, Kiyoshi (author) is a Medicine article available to read on EtoBox.

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## Abstract ## Background Schnitzler’s syndrome (SchS) is a rare autoinflammatory syndrome with diagnostic challenge and be characterized by chronic urticaria, a monoclonal gammopath, periodic fever and bone pain. In addition to the monoclonal gammopathy, bone abnormalities are often found at the site of bone pain in patients with SchS. The remarkable efficacy of interleukin-1 (IL-1) inhibition was also demonstrated in this syndrome. ## Case presentation We describe a case of refractory chronic urticaria presenting with clinical manifestations consistent with SchS without monoclonal gammopathy. A 43-year-old female patient suffering from recurring of urticaria with periodic fever as well as bone pain for the past 4 years. The patient had leukocytosis and elevated levels of C-reactive protein (CRP) and serum amyloid A (SAA). PET/CT (positron emission tomography/computed tomography) and MRI (magnetic resonance imaging) examination revealed hyper-metabolism areas in both femoral bone marrow. Although bone marrow histology revealed no abnormality, urticarial skin lesions shows neutrophilic infiltrations without evidence of vasculitis. We could not exclude the possibility of SchS. The p

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Author

Fujita, Yuya (author);Asano, Tomoyuki (author);Sakai, Akira (author);Norikawa, Natsumi (author);Yamamoto, Toshiyuki (author);Matsumoto, Haruki (author);Sato, Shuzo (author);Temmoku, Jumpei (author);Yashiro-Furuya, Makiko (author);Matsuoka, Naoki (author);Watanabe, Hiroshi (author);Migita, Kiyoshi (author)

Publisher

Springer Science and Business Media LLC

Published

2021

Language

EN

Field

Medicine (Health Sciences)